Late survival in Ellis-van Creveld syndrome with common single atrium.

ABSTRACT: Ellis-van Creveld (EVC) syndrome is a relatively rare chondroectodermal dysplasia considered mainly as a generalised disorder of the maturation of endochondral ossification. Congenital heart disease occurs in approximately half of the patients with EVC syndrome, 60% of which is a common atrium. Common atrium is a rare variety of interatrial communication characterised by absence or virtual absence of the atrial septum. Patients with this syndrome rarely survive to an old age. We report a case of EVC who presented for the first time at 60 years of age, as survival to an advanced age is exceptional in case of EVC.

SUBMITTER: Prajapati K 

PROVIDER: S-EPMC7993251 | biostudies-literature | 2021 Mar

REPOSITORIES: biostudies-literature